We report a case of a 44-year-old woman with isolated neurosarcoidosis with florid craniospinal leptomeningeal enhancement. Her main symptoms of headache, diplopia, ataxia and perineal numbness failed to respond to prednisolone, azathioprine and methotrexate. Following two induction doses of infliximab there was symptomatic improvement with marked resolution of radiological changes. Infliximab (5 mg/kg) given every 8 weeks with methotrexate 15 mg weekly sustained remission for 2 years, then symptoms and radiology relapsed. Eleven cases of neurosarcoidosis responding to infliximab are reported. Infliximab is a monoclonal antibody against the pro-inflammatory cytokine TNF-α which is involved in the granulomatous inflammation of sarcoidosis. There is increasing evidence of its efficacy in the treatment or refractory sarcoidosis. Immunogenicity with antibody formation limits the long-term use of this agent but reducing dose intervals and increasing doses may counteract this.